{
  "abstract": "Objectives To characterize systemic lupus erythematosus (SLE) patients with Hemophagocytic lymphohistiocytosis (HLH) in a tertiary Rheumatology centre.Methods Retrospective descriptive analysis of SLE patients who developed HLH and were admitted to the Rheumatology Department between 2000–2025. All patients met the 2019 EULAR/ACR Classification Criteria for SLE, fulfilled the HLH-2004 diagnostic criteria and had an HScore >169 points.Results Three female patients were identified ( table 1). Case 1: A 27-year-old female with SLE, who was on hydroxychloroquine (HCQ) and rituximab (RTX), was admitted with an axillary abscess. She developed fever and a progressively obnubilated mental state. Laboratory tests showed pancytopenia, elevated liver enzymes, hyperferritinemia, hypertriglyceridemia, low complement, and high anti-dsDNA. Blood cultures were positive. CT: lymphadenopathies and hepatomegaly. Despite antibiotics, intravenous immunoglobulin and high dose steroids, her condition worsened, resulting in multiorgan failure, with fatal outcome. Case 2: A 32-year-old female was admitted with fever and proteinuria. She developed altered consciousness and marked peripheral oedema. Laboratory evaluation revealed pancytopenia, hyperferritinemia, elevated liver enzymes, hypertriglyceridemia, positive antinuclear antibodies, high anti-dsDNA and low complement. CT: lymphadenopathies and hepatosplenomegaly. A kidney biopsy revealed a class IV lupus nephritis. She was diagnosed with new-onset SLE and probable HLH and started on high dose steroids and RTX, leading to marked improvement. Case 3: A 40-year-old female with SLE on HCQ presented with fever, severe malaise and mental confusion. Blood tests demonstrated thrombocytopenia, markedly elevated ferritin, severe transaminitis and low fibrinogen. CMV IgM antibodies were positive. CT: lymphadenopathies and hepatomegaly. She was treated with anakinra and low dose steroids, resulting in rapid improvement. Bone marrow biopsy was not performed in any of the patients.Abstract PO:04:121 Table 1Disease characteristics of the three casesConclusions In line with previous reports, all cases in our series were temporally related to lupus activity or infections, presenting with fever, cytopenias, and other typical HLH features. Hyperferritinemia was common, with ferritin >1000 ng/mL, highlighting its value in suspecting HLH in SLE. Management included steroids with additional immunosuppressive agents depending on severity. In case 1, due to severe infection, immunosuppressive agents were avoided, and antibiotic and supportive care were intensified. HLH in SLE may mimic disease flare. Early recognition, triggers identification, and timely treatment improve prognosis.",
  "authors": [
    {
      "affiliations": [
        "Rheumatology department, Unidade Local de Saúde do Alto Minho, Ponte de Lima, Portugal"
      ],
      "name": "Susana Almeida"
    },
    {
      "affiliations": [
        "Rheumatology department, Unidade Local de Saúde do Alto Minho, Ponte de Lima, Portugal"
      ],
      "name": "Anita Cunha"
    },
    {
      "affiliations": [
        "Rheumatology department, Unidade Local de Saúde do Alto Minho, Ponte de Lima, Portugal"
      ],
      "name": "Diana Barros"
    },
    {
      "affiliations": [
        "Rheumatology department, Unidade Local de Saúde do Alto Minho, Ponte de Lima, Portugal"
      ],
      "name": "Maria Pontes Ferreira"
    },
    {
      "affiliations": [
        "Rheumatology department, Unidade Local de Saúde da Guarda, Guarda, Portugal"
      ],
      "name": "Duarte Augusto"
    },
    {
      "affiliations": [
        "Rheumatology department, Unidade Local de Saúde do Alto Minho, Ponte de Lima, Portugal"
      ],
      "name": "José Tavares-Costa"
    },
    {
      "affiliations": [
        "Rheumatology department, Unidade Local de Saúde do Alto Minho, Ponte de Lima, Portugal"
      ],
      "name": "Filipa Teixeira"
    },
    {
      "affiliations": [
        "Rheumatology department, Unidade Local de Saúde do Alto Minho, Ponte de Lima, Portugal"
      ],
      "name": "Soraia Azevedo"
    },
    {
      "affiliations": [
        "Rheumatology department, Unidade Local de Saúde do Alto Minho, Ponte de Lima, Portugal"
      ],
      "name": "Daniela Peixoto"
    },
    {
      "affiliations": [
        "Rheumatology department, Unidade Local de Saúde do Alto Minho, Ponte de Lima, Portugal"
      ],
      "name": "Francisca Guimarães"
    }
  ],
  "title": "PO:04:121 Systemic lupus erythematosus – associated hemophagocytic lymphohistiocytosis: a difficult diagnosis",
  "uid": "3f5a7420-7dd3-554e-a221-80bced75ba46"
}